Autosomal dominant hereditary disorder	Developmental absence of tooth	Hereditary cancer-predisposing syndrome	Oligodontia
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Oligodontia and cancer predisposition syndrome (disorder)
	Oligodontia and cancer predisposition syndrome
	A rare genetic odontologic disease with characteristics of the congenital absence of six or more permanent teeth (excluding the third molars) in association with an increased risk for malignancies, ranging from gastrointestinal polyposis to early-onset colorectal cancer and/or breast cancer. Ectodermal dysplasia (manifesting with sparse hair and/or eyebrows) may also be associated.
	syndroom van oligodontie en predispositie voor maligne neoplasma
	syndroom van oligodontie en predispositie voor maligniteit

Id	773345007
Status	Primitive

Associated morphology	Absence
Finding site	Dentition
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Congenital abnormal number
Finding site	Tooth structure
Occurrence	Congenital
Pathological process	Pathological developmental process

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	K00.0
Rule	TRUE
Advice	ALWAYS K00.0 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified